Background
To describe a rare case of isolated noncompaction cardiomyopathy and stroke and to
review the medical literature on noncompaction cardiomyopathy.
Methods
Retrospective chart review of the case was performed. Extensive literature review
on etiology, clinical presentation, diagnosis, and management of noncompaction cardiomyopathy
was also performed.
Results
Our patient is a healthy 20-year-old woman who presented with sudden onset left face
and arm weakness and hypesthesia. Magnetic resonance imaging (MRI) brain showed right
middle cerebral artery (MCA) infarct. Magnetic resonance angiography head showed right
MCA artery (M2) cutoff. MRI neck was nonsignificant. Echocardiogram was suggestive
of noncompaction of left ventricle. Cardiac MRI confirmed the noncompaction of the
left ventricle myocardium, which was thought to be the etiology of stroke. Patient
was started on anticoagulation for secondary stroke prevention.
Conclusions
Isolated left ventricular noncompaction cardiomyopathy (LVNC) is a rare form of primary
genetic cardiomyopathy, which occurs because of the arrest of the process of compaction
of ventricular myocardium during embryogenesis. Noncompaction cardiomyopathy is usually
associated with other primary cardiac structural abnormalities like dysfunctional
cardiac valves. In isolated noncompaction cardiomyopathy, there are no other primary
cardiac structural abnormalities. The most common clinical features seen in LVNC include
left ventricular systolic dysfunction, congestive heart failure, arrhythmias, and
cardiac embolic events theorized to result from thrombus formation within the intertrabecular
recesses. As it is a rare disease, evidence-based recommendations for preventing thromboembolic
events in isolated left ventricular noncompaction have not been established.
Key Words
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Article info
Publication history
Published online: June 03, 2015
Accepted:
April 12,
2015
Received:
February 16,
2015
Footnotes
A.K. contributed to article design, drafting, and revision and figure selection. N.K. contributed to article drafting. C.S. contributed to study supervision and article editing.
This is a nonfunded, nonsponsored study. All authors report that they have no disclosures.
Identification
DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2015.04.014
Copyright
© 2015 National Stroke Association. Published by Elsevier Inc. All rights reserved.
